Idioma:

Genetic disruption of both Fancc and Fancg in mice recapitulates the hematopoietic manifestations of Fanconi anemia

Pulliam-Leath, Anna C. ; Ciccone, Samantha L. ; Nalepa, Grzegorz ; Li, Xiaxin ; Si, Yue ; Miravalle, Leticia ; Smith, Danielle ; Yuan, Jin ; Li, Jingling ; Anur, Praveen ; Orazi, Attilio ; Vance, Gail H. ; Yang, Feng-Chun ; Hanenberg, Helmut ; Bagby, Grover C. ; Clapp, D. Wade

Blood, 2010-10, Vol.116 (16), p.2915-2920 [Periódico revisado por pares]

Washington, DC: Elsevier Inc

Texto completo disponível

Citações Citado por

Enviar para

Título:
Genetic disruption of both Fancc and Fancg in mice recapitulates the hematopoietic manifestations of Fanconi anemia
Autor: Pulliam-Leath, Anna C. ; Ciccone, Samantha L. ; Nalepa, Grzegorz ; Li, Xiaxin ; Si, Yue ; Miravalle, Leticia ; Smith, Danielle ; Yuan, Jin ; Li, Jingling ; Anur, Praveen ; Orazi, Attilio ; Vance, Gail H. ; Yang, Feng-Chun ; Hanenberg, Helmut ; Bagby, Grover C. ; Clapp, D. Wade
Assuntos: Anemias. Hemoglobinopathies ; Animals ; Biological and medical sciences ; Bone Marrow - physiopathology ; Chromosome Aberrations ; Diseases of red blood cells ; Fanconi Anemia - genetics ; Fanconi Anemia Complementation Group C Protein - genetics ; Fanconi Anemia Complementation Group G Protein - genetics ; Hematologic and hematopoietic diseases ; Hematopoiesis and Stem Cells ; Leukemia, Myeloid, Acute - genetics ; Medical sciences ; Mice ; Mice, Inbred C57BL ; Mutation ; Myelodysplastic Syndromes - genetics
É parte de: Blood, 2010-10, Vol.116 (16), p.2915-2920
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
A.C.P.-L. and S.L.C. contributed equally to this study.
Descrição: Fanconi anemia (FA) is an inherited chromosomal instability syndrome characterized by bone marrow failure, myelodysplasia (MDS), and acute myeloid leukemia (AML). Eight FA proteins associate in a nuclear core complex to monoubiquitinate FANCD2/FANCI in response to DNA damage. Additional functions have been described for some of the core complex proteins; however, in vivo genetic proof has been lacking. Here we show that double-mutant Fancc−/−;Fancg−/− mice develop spontaneous hematologic sequelae including bone marrow failure, AML, MDS and complex random chromosomal abnormalities that the single-mutant mice do not. This genetic model provides evidence for unique core complex protein function independent of their ability to monoubiquitinate FANCD2/FANCI. Importantly, this model closely recapitulates the phenotypes found in FA patients and may be useful as a preclinical platform to evaluate the molecular pathogenesis of spontaneous bone marrow failure, MDS and AML in FA.
Editor: Washington, DC: Elsevier Inc
Idioma: Inglês

Links

Voltar para lista de resultados

Realização: Logos de Redes Sociais:

Genetic disruption of both Fancc and Fancg in mice recapitulates the hematopoietic manifestations of Fanconi anemia

Pulliam-Leath, Anna C. ; Ciccone, Samantha L. ; Nalepa, Grzegorz ; Li, Xiaxin ; Si, Yue ; Miravalle, Leticia ; Smith, Danielle ; Yuan, Jin ; Li, Jingling ; Anur, Praveen ; Orazi, Attilio ; Vance, Gail H. ; Yang, Feng-Chun ; Hanenberg, Helmut ; Bagby, Grover C. ; Clapp, D. Wade

Washington, DC: Elsevier Inc

Buscando em bases de dados remotas. Favor aguardar.